Gigantiform melanotic neuroectodermal tumor of infancy

Melanotic neuroectodermal tumor of infancy is a rare neoplasm of possibly neural crest origin, and it predominantly occurs in the premaxillas of infants less than 12 months old. Generally, the treatment of this benign pigmented lesion is conservative surgical excision. Long-term follow-up is imperative inasmuch as local recurrence and malignant transformation have been reported. A case with exceptional clinical features involving a 7-month-old child is presented.